Flashcards in Kaplan Questions Deck (108):
Describe the rash associated with erythema infectiosum
aka Fifth disease is a viral infection by human parvovirus B19 that is mildly contagious and affects children 3-12 yrs. An erythematous malar rash appars --> erythematous maculopapular eruption on the extensor extremities the next day. When the rash fades a few days later, areas of central clearing leave behind a reticulated, lacy patterned rash thatwaxes and wanes for an avg of 9-11 days.
Wiskott aldrich is an X-linked disease characterized by a triad of eczema, thrombocytopenia and recurrent infections. Child has increased risk of?
Lymphoma inf they survive past 15 since they often have severe infections
Gaucher disease -AR lysosomal storage disease caused by deficiency of glucocerebrosidase (aka glucosylcermidase): what are the symptoms?
-asymptomatic painless HSM possibly with elevated transaminases
-BONE abnormalities (pain crisis, avascular necrosis, osteoporosis, fractures and even abnormal modeling with widening of the distal femurs looks like ERLENMEYER FLASK!)
JIA -worry about which most feared complication?
do slit lamp to screen for iridocyclitis
Which congenital heart disease is assoc with?
-a single S2
-CXR: normal heart size and increased pulmonary vascular markings ("SNOWMAN")
-EKG: RVH, peaked p waves
-ABG: respiratory acidosis
Total anomalous pulmonary venous return when pulmonary veins drain into the right atrium --> right atrial enlargement (spiked p waves)
IF you suspect shaken baby syndrome, what should be done before reporting to CPS?
bleeding disorders should be ruled out so get coag studies
Acute osteo is inflammation of bone marrow caused by infection that is rapid and usu severe. Symptoms include fever, refusal to bear weight or ambulate with a limp and localized pain and tenderness. Most common causative agent in children?
most commonly via hematogenous spread
When a premature infant develops obstructive hydrocephalus with symptoms like poor feeding, irritability, reduced activity, tense anterior fontanelle, distended scalp veins, sunset sign (eyeballs are deviated downward and upper lids are retracted), suspect!?
Germinal matrix hemorrhage aka intraventricular hemorrhage. The GM is a highly vascularized layer of neuroectodermal precursors lining the ventricular cavities. These vessels are very vulnerable to hypoxic insults.
Kawasaki Disease aka mucocutaneous LN syndrome -a systemic vasculitis of unknown etiology. List symptoms and treatment and what to worry about
-fever >/- 5 days often to 104
-b/l nonexudative conjunctival injection
-changes of the oral mucosa, injected pharynx, or strawberry tongue
-polymorphic exanthem of the trunk
-changes in hands and feet including redness, swelling
Do screen for heart conditions like coronary artery aneurysms
Treat with IVIG and high-dose aspirin
Which acid-base condition is assoc with pyloric stenosis?
vomiting so metabolic alkalosis
Cystinuria -AR d/o affecting reabsoprtion of COAL (cystine, ornithine, arginine, lysine) --> urolithiasis
What will you see on UA? How to diagnose?
Hexagonal crystals on UA are characteristic of cystine crystals. And dx is confirmed with a 24-h urine for cystine
How to differentiate btw congenital hemophilia A with low factor VIII levels vs acquired hemophilila A 2/2 to anti-factor VIII abs?
plasma mixing study
-add plasma with factor VIII into pt's serum
-if PTT improves, then it's congenital, but if it doesn't, then it's acquired bc the added VIII will still be attacked by the antibodies
vWF deficiency -how to diagnose? This is defect in both plt aggregation as vWF acts like a glue to bind plts to endothelium and clotting factor activity (as vWF is the cofactor of factor VIII). What will you see in labs?
Ristocetin cofactor activity -tests the ability of vWF to agglutinate plts by binding to its primary receptor, GP1b
Bleeding time is prolonged b/c of decreased plt functions
Increased PTT b/c vWF serves as cofactor for VIII
Jones Criteria for Rheumatic Fever: Dx requires 2 major or 1 major + 2 minor symptoms
Major symptoms: Carditis, Erythema marginatum and subcutaneous nodules, Sydenham's chorea, arthritis
Minor symptoms: Fever, polyarthralgia, reversible prolongation of the PR interval, Increased ESR, recent beta-hemolytic strep infection, h/o of rheumatic fever
T1DM has increased risk of which GI disease?
-should get IgA and tissue transglutaminase antibody
Ebstein anomaly -what with CXR show
ebstein anomaly is when there's a hypoplastic RV and enlarged RA b/c of tricuspid atresia. CXR will show cardiomegaly and and decreased pulmonary vasculature
Chronic granulomatous disease -def of NADPH oxidase that causes inadequate production of H2O2, superoxide and other O2 species in neutrophils. What's the test?
nitroblue tetrazolium dye reduction test will confirm defective neutrophils inability to produce these o2 species necessary in killing of bacteria (staph, burkholderia, pseudomona, serratia, nocardia, aspergillus)
What does monoclonal anti-CD11 help diagnose?
LAD deficiency in which the CD11 antigen on surface of WBCs is missing. This can lead to delayed cord healing
What's the most common bacterial infection causing CNS infections in those who have neurosurg procdures?
staph epidermidis produces biofilm that allows them to adhere to surfaces of medical prostheses.
will need vancomycin
Roseola infantum due to HHV6 and 7
maculopapular rash that spreads from trunk to extremities and is assoc with resolution of high fevers
If you discover a mass of SCM muscle after birth -it is most commonly?
congenital torticollis aka SCM tumor of infancy that will regress in 4-8 months
freckling in axillary region
osseous lesion/BONY dysplasia
Tuberous sclerosis: symptoms
ash-leaf macules (hypopigmented macules)
facial angiofibromas (adenoma sebaceum)
Shagreen patches (CT nevi)
Seizures (most common presenting symptom)
Sturge Weber: symptoms
cerebral calcificatiosn (train tracks)
port wine stain
glaucoma in ipsilateral eye
seizures c/l to side of nevus
made with skull x-ray or CT showing calcifications
What's the test to screen for scoliosis? What's to diagnose?
Adams test: have the patient forward bend and look at spine.
X-ray to diagnose
Activities observed in a 1-2 month old
-holds head erect and lifts head alone
-turns from side to back
-visually follows objects
-alert to voices
Activities observed in a 3-5 month old
-reaches for objects and brings them to mouth
-sits with support
-turns from back to side
Activities observed in a 6-8 month old
-sits alone and without support
-rolls from back to stomach
Activities observed in a 9-11 month old
-walks by holding furniture for support
-follows one step commands: "come here", "give it to me"
Activities observed in a 1 year
-tower of 2 cubes
-points to desired objects
-says one or 2 other words than mama/dada
Activities observed in 18 months
-builds a tower of 3-4 cubes
-walks up and down stairs with help
-says 4-20 words
Activities observed in 24 months
-throws a ball overhead
-walks up and down stairs one at a time
-jumps and climbs
-helps to undress only
-listens to stories well
-knows 50 words and can put 3 words together
Activities observed in 3 yr-olds
-holds crayons with fingers
-dresses with supervision
Children tolerate SVTs (180-300 bpm) very a short period of time well, only complaining about heart beating fast. Chest pain and heart failure can result if in SVT for a long time. What are some conditions that predispose one to SVT/are assoc with SVT? How to treat?
-uncorrected congenital heart defect like Ebstein anomaly
-WPW (short PR and slow upstroke of QRS)
Treat by submerging face in iced saline, vagotonic maneuvers
If that doesn't work, then try rapid IV push of adenosine
If heart failure is present, then synchronized DC cardioversion
Growing pains are most commonly bilateral, involve the lower leg and knees, manifest with deep pain during rest (usu at bedtime). How to treat?
massage, heat, simple analgesic therapy
Presence of what abs implies an excellent prognosis in pts with JIA
rash characterized by petechiae which become --> Lower-extremity purpura, arthritis, abdominal pain, hematuria -think!!??
Henoch Schonlen Purpura
H. Influenzae is a gram negative, oxidase positive coccobacillus that grows on ___ agar?
The double bubble sign on x-ray is pathognomonic for?
Asplenic pts are more prone to infections with encapsulated bacteria like?
Pelvic exam showed erythematous cervix ("strawberry cervix") with punctuate hemorrhage, and a frothy green d/c without cervical motion tenderness, foul-smelling discharge. What's the most likely cause? Most approp next diagnostic test?
Vaginal discharge microscopy can show motile trichomonads on wet mount w/ vaginal pH > 4.5
Treat with metronidazole
Esophageal atresia presents with vomiting and coughing after feeds in the first 24 hr after birth, and is assoc with what on xray?
hep c + mothers -can they breastfeed
yes! no contraindication
HUS is most common cause of acute renal failure in young children and most frequently follows an episode of gastroenteritis caused by EHEC O157:H7. Pathogenesis? Treatment?
Endothelial injury to kidneys --> renal failure
damage to red cells and plts --> thrombocytopenia and microangiopathic hemolytic anemia
supportive care, but if renal disease is really bad with marked elevation of k and creatinine, start dialysis
"egg on a string appearance" is talking about which congenital heart condition?
Transposition of the great vessels b/c aorta comes from RV and pulmonary artery from LV, which will make the pulmonary artery appear absent giving the cardiac silhouette the appearance of "an egg on a string"
this is ductus-dependent so give prostaglandin E to keep it OPEN. An arterial switch operation is needed
If you suspect tracheoesophageal fistula, what should be done before surgery to confirm?
nasogatric tube should be passed to confirm the diagnosis.
Most serious complications of sinusitis and otitis?
cerebral abscesses (order head CT or MRI)
manifests with persistent fever, variable neurologic deficits, headache, seizures, increased intracranial pressure
Mumps is characterized by a prodrome of low-grade fever, malaise, headache, myalgias, and anorexia. Parotitis is caused by direct infection of stenson duct. what is the most common comp of mumps in prepubertal children vs adults?
prepubertal: meningoencephalomyelitis caused by spread of infection in CNS
Anal pinworms caused by enterobius vermicuaris confirmed by scotch tape test. Treatment?
albendazole or mebendazole
single dose, followed by a second dose 2 wks later
Neuroblastoma is a malignancy of neural crest cells that present as an abdominal mass and located either in the adrenal gland or along the sympathetic chain. It tends to encase and surround abdominal structures. What will CT scan show? How about MIBG scan?
CT scan of abdomen will show an extrarenal tumor. MIBG scans use radiotracer that binds to NE receptors that will identify neuroblastomas
Necrotizing enterocolitis is caused by local ischemia, dilatation and infarction of loops of bowel. It can be superinfected. Premature infants are more susceptible. What on imaging confirms the diagnosis?
clinically: abd distension, hematochezia or occult blood
imaging: pneumatosis intestinalis
Infantile hemangiomas are most common benign tumor of childhood that may occur cutaneously or be present within any organ. They are usu self-limited and begin involuting after 18 months. However, there are some complications that can arise like?
-high output cardiac failure resulting from increased blood flow to the lesions, esp in hemangiomas are in the liver
-plt sequestration and destruction leading to coagulopathy
-can rupture and bleed
Treatment includes steroids and interferon
Breast milk jaundice vs breastfeeding jaundice
Breast milk jaundice: an inhibitor of glucuronyl transferase is presentin breast milk of some mothers --> indirect hyperbilirubinemia, but doesn't increase until 2nd week of life; d/c breastfeeding for 24-48 hrs, drink formula, let the bilirubin drop, and then try breastmilk again
Breast feeding jaundice: does not get suff calories --> dehydration --> increase in enterohepatic circulation of bilirubin --> indirect hyperbilirubinemia; seen in 2nd-4th day of life; treatment: re-hydrate and get mom lactation consult
Bone fragility, short stature, scoliosis, blue sclerae, hearing loss, increased laxity of the ligaments and skin, easy bruisability. Suspect?
Identify the murmur: mid-systolic click followed by a late systolic murmur
mitral valve prolapse -can produce subjective chest pain and palpitations
mid-diastolic murmur heard after an opening snap
Acute rheumaticfever is diagnosed based on evidence of Group A b-hemolytic strep infection with the jones criteria (major: migratory polyarthritis, carditis, subcutaneous nodules, erythema marginatum, syndenhamchorea; minor: fever, athralgia, lab abnormalities, prolonged PR, previous rheumatic fever). What's the most common complication of rhuematic fever?
valvular disease -mitral valve most likely --> aortic --> tricuspid --> pulmonary
acute encephalopathy assoc with high ammonia levels. It occurs in children after a viral illness and risk is increased when aspirin is given. Classic features are rash, vomiting, and hyperammonemia. It causes fatty infiltration of the liver and cerebral edema
UC is a chronic inflamm disease that is continuous and almost always involves the rectum. Bloody diarrhea with or w/o mucus, rectal urgency and tenesmus are common. Increased risk of cancer so colonoscopies should be done after 10 yrs of having disease. Which antibody is assoc with disease?
Hypoplastic left heart syndrome when there's atresia or stensois of mitral and aortic valves. A patent foramen ovale should divert pulmonary venous blood from LA to RA leading to dilated and hypertrophied right side. A PDA is needed to divert pulmonary venous blood that is now in the right side from pulmonary artery to aorta to systemic circulation. Giving O2 rarely improves the clinical situation b/c O2 will cause pulmonary vessels to dilate and pools the blood so less is going to systemic circulation. What do you expect to hear and see on imaging and ekg
hear: hyperdynamic precordium b/c enlarged right ventricle is contracting against systemic pressure with a LOUD or even palpable second heart sound (S2) b/c of increased blood coursing thru pulmonary artery
EKG: RAD, and right atrial and ventricular hypertrophy
CXR: as it worsens will have marked cardiomegaly and increased pulmonary vascular markings
ECHO: is diagnostic
Osgood-Schlatter disease is a common cause of anterior knee pain in the adolescent. Pathogenesis?
It is a stress reaction of the insertion of the patellar tendon into the tibial tubercle causing osteochondritis.
suspect in young teenager who is active in sports that complaisn of pain and swelling over the tibial tubercle.
Imaging can be normal
GU anomalies (cryptochidism, ambig genitalia)
Deletion in chromosome 11
Treatment of choice for strep pharyngitis?
oral penicillin V for 10 days
if concern for non-compliance or lost to follow-up, can do single-dose benzathine penicillin G
Neonatal listeriosis can cause what change to the amniotic fluid? What's pathognomonic for neonatal listeriosis?
Neonatal listeriosis can cause amnionitis which is characterized by brown, murky amniotic fluid. It can cause neonatal sepsis with GRANULOMATOSIS INFANTISEPTICA which is pathognomonic.
What's the treatment for bronchopulmonary dysplasia? What to worry about?
Furosemide -can lead to dehydration with electrolyte abnormalities (hypokalemia/metabolic alkalosis)
Most common cardiac abnormality in infants of diabetic mothers is?
asymmetric septal hypertrophy that will improve over time
w/ significant hypertrophy, there would be decreased perfusion as evidenced by weak pulses, decreased blood pressure, and prolonged cap refill.
A hamartoma that presents as a sharply circumscribed verrucuous plaque that is present at birth on scalp (but can be found on face or neck) without hair that grows and undergoes changes (becomes more yellow and warty). What is it?
Nevus sebaceous of Jadasohn -that can develop into a malignancy so impt to excise at an early age
Anuria within the first 24 hrs of life in a male infant -suspect?
Posterior urethral valves
next step is to place a urethral catheter to drain the bladder.
Voiding cystourethrogram is the single best diagnostic test to confirm the presence of urethral valves
If confirmed, then can be surgically removed
Valproic acid exposure in utero can lead to what types of deformities
neural tube defects like meningomyelocele
Isotretinoin exposure in utero can lead to what types of deformities
CNS defects like hydrocephalus, microcephay,
Early onset sepsis vs late onset neonatal sepsis
Early onset: birth - 7 day
Late onset: 8-28 days
causative bacterial agents: GBS, listeria, e. coli
occurs in 80% of infants if mother is infected during 1st trimester
-heart defects like PDA
-blueberry muffin (signs of extramed hematopoiesis)
dx: IgM or culture
Apnea of prematurity (cessation of air flow/exchange > 20 seconds) is often assoc with bradycardia and hypoxemia. What's the first-line pharmacologic agents?
methylxanthines (caffeine and theophylline) which act to stimulate respiratory neurons
Endocardial cushion defect is the most common heart defect assoc with down syndrome. What is it?
when there is a ASD and VSD that are present and contiguous. Can be found to have systolic thrill
How to manage subsequent pregnancies of a mother who gave birth to a child with congenital adrenal hyperplasia?
give dexamethasone which will cross placenta to suppress fetal adrenal steroid secretion to prevent masculinization.
CVS is then performed to determine the genotype
Intussusception when portion of GItract slips or telescopes into the portion just distal to it -most commonly ileocolic. Clinical symptoms? Best way to diagnose?
Clinically: episodes of distress and crying interspersed with quiet periods of normal behavior and playing. Acute onset of cramping and colicky abdominal pain.
As obstruction progresses,may develop fever and lethargy. Assoc with stool mixed with blood and mucus aka "currant-jelly" stool.
An air contrast enema is both diagnostic and therapeutic and is treatment of choice for symp less than 48 hrs
If longer, surgery is necessary bc of risks of perforation, peritonitis
Developmental displasia of the hip describes a spectrum of conditions in which the femoral head has an abnormal relationship to the acetabulum resulting in hip instability. Risk factors include?
limited fetal mobility (1st born b/c less space in primigravida uterus)
Very low birth weight infants are at high risk for cardiac defects, pulmonary immaturity, intraventricular hemorrhage, necrotizing enterocolitis, infections and other complications. Whats the most common?
Bacterial vaginosis is characterized by excessive discharge and odor seen predom in sexually active premenopausal women, but is not a STD. Causative agents like gardnerella vaginalis. How to diagnose?
-pH of vaginal d/c is > 4.5
-one drop of 10% KOH is added to one drop of discharge --> an intense amine odor = "positive whiff-test"
-on NS wet prep: d/c has a predominance of clue cells
-treat with metronidazole
Congenital hypothyroidism aka cretinism: coarse facial features, enlarged fontanelles, umbilical hernia, mental retardation, scares lanugo, weak cough, jaundice, hypotonia, constipation. Full clinical pic develops by 3-6 months of age. Most commonly due to?
McArdle disease -muscle phosphorylase deficiency; which is clinical presentation
present in adolescence with exercise intolerance with fatigue, myalgia, cramps, myoglobinuria, msucle swelling and fixed weakness
What is transient erythroblastopenia of childhood? How to manage?
most commonly acquired, self-limited form of pure red-cell aplasia that is usually preceded by a viral infection in children btw 6 mo and 3 years. Symptoms include gradually increasing pallor and fatigue, tachycardia and cardiac flow murmur.
management includes follow-up of Hb levels until resolution
How to differentiate btw transient erythroblastopenia of childhood (self-limited, most commonly acquired pure cell aplasia) and congenital hypoplastic anemia aka diamond-blackfan anemia?
adenosine deaminase levels are normal in transient erthroblastopenia while it's elevated in congenital hypoplastic anemia
What should be performed on sisters of males with Fragile X?
Cytogenetic testing b/c heterozygous femaleshave developmental and behavioral problems like ADHD, and/or borderline or mild mental retardation
Person from mexico with 1-2 wks of fever and RUQ abd pain with elevated liver function tests and leukocytosis. Ultrasoundis the first imaging study to perform and shows an abscess. What is it? What's the next thing to order?
most likely amebic liver caused by infection with entameoba histolytica. Next is to look at serum antibodies. Treatment is metronidazole.
Definitive surgical treatment for PDA
Coxsackie myocarditis in a newborn presents like
shock-hypotension, poor perfusion, metabolic acidosis, distant heart sounds, loud apical murmur, cardiomegaly and pulmonary edema.
What is the first step in management of congenital diaphragmatic hernia
nasogastric suction for bowel decompression to prevent further lung compression and immediate intubation
How to treat acute rheumatic fever?
penicillin and and aspirin
Staphylococcal scalded skin syndrome occurs in children younger than 5 y.o. Beginning of the illness is characterized by constitutional symptoms like skin tenderness, followed by a diffuse scarlatiniform erythema with flaccid blisters and erosions. + Nikolsky sign. HOw to treat?
IV anti-staph antibiotics like oxacillin or nafcillin
When CF is suspected, treat the acute severe exacerbation of pulm infection with immediate hosp and IV abx therapy. Which is the most commonly used regimen?
IV tobramycin (great gram negative andpseudomona coverage) with ceftazidime (covers pseudomona as well as well as gram+ and gram -)
transient synovitis aka postviral synovitis occurs 1-2 weeks after a respiratory infection or getting?
a rubella vaccine
Endocarditis (subacute-strep viridans, acute -staph) -is confirmed with/most impt piece in establishing diagnosis?
positive blood cultures
other signs like oslernodes (tender nodules on the finger and toe pads) and janeway lesions (painless hemorhagic lesions on the palms and soles), splinter hemorrhages (linear lesions beneath the nails) are just symptoms
which congenital heart disease is most common to present with cyanosis in the first 24 hours of life?
transposition of great vessels
give IV PGE1 to keep ductus open
Adrenoleukodystrophy is an X-linkeddisease that results from mutations that prevent transport of veryLCFA into peroxisomes preventing b-oxidation and breakdown of LCFA. what are clinical symptoms
neuro deficits begins with weakness and spasticity then dementia, blindness and quadriparesis
adrenal insufficiency: hyponatremia, postural hypotension, hyperkalemia, lack of response to consyntropin stimulation test
Aortic stenosis in children is mostly caused by a congenital bicuspid valve. Describe murmur
systolic ejection murmur (crescendo-decrescendo systolic murmur that peaks in midsystole and radiates into the carotids) that is loudest at the right upper sternal border
In immunocompromised folks, what's a serious complication to look out for if they have the chicken pox?
If you suspect choanal atresia, the initial step is pass catheter thru nose. But what is the confirmatory test?
CT scan of head with intranasal contrast to show the narrowing
When do you need surgery for a baby with necrotizing enterocolitis?
When there's perforation (free air on left lateral decubitus or under the diaphragm) or fixed dilated loops of bowel abdominal wall cellulitis, clinical deterioration.
Most common organismsisolated from a cat bite are? What's the first line treatment?
-pasteurella multocida and staph aureus
Neonatal resuscitation involves what steps
1) place the infant on the radiant warmer
2) dry and stimulate the infant to breathe
3) open the airway and provide positive pressure ventilation (bag-mask) with oxygen)
Which two reflexes are the first to disappear
palmar and rooting reflexes
once serologically confirmed, what's the abx of choice to treat lyme disease in someone younger than 6?
Osteoid osteoma usu affects pts btw ages 5-24 y.o, affecting LE and the spine with pain worse at night that is relieved with aspirin. What will x-rays usu show?
a sclerotic lesion and sometimes a localized lytic defect or nidus within the sclerotic bone. Treatment involves surgical excision.
Ewing sarcoma is malignant bone tumor of bone arising in medullary tissue. Symptoms include pain, fever and leukocytosis. what will x-rays show?
"onion skin appearance"
Empiric treatment for neonatal sepsis? what if neonatal meningitis?
neonatal sepsis: ampillcilin + gentamicin
neonatal meningitis: cefotaxime + amp
Congenital varicella symptoms. how to prevent?
limb hypoplasia, cutaneous scars, cataracts, chorioretinitis, and cortical atrophy.
Can give VZIG if mom is exposed 5 days before and 2 days after delivery.